Weber syndrome

The Weber syndrome is a form of brain stem syndrome. Often it is due to an ischemic stroke as a result of a thromboembolism. Typical consequences are unilateral paralysis, impaired eye motor skills and other neurological damage.

What is Weber Syndrome?

The Weber syndrome belongs to the brainstem syndromes, which all go back to damage in the brain area of ​​the same name. The doctor David Weber first described the clinical picture, which is also called Midbrain syndrome is known. Damage to the substantia nigra in the midbrain and the interruption of certain nerve tracts are characteristic of Weber syndrome.

The main affected areas are the corticopontinus tract, which is connected to the bridge and the cerebral cortex, the oculomotor nerve, which controls eye movements, and the pyramidal tract, which transmits motor signals to the body. The substantia nigra is a core area in the midbrain that has numerous connections to other areas of the brain.

These include, for example, the striatum, thalamus, globus pallidus, nucleus subthalamicus and the cerebral cortex. The substantia nigra also plays an important role in its function within the extrapyramidal motor system. This system controls movements like the pyramidal orbit.

causes

Weber syndrome can have various causes. In the majority of cases, the clinical picture comes about as a result of an ischemic stroke, in which one or more blood vessels that lead to the brain become blocked. As a result, the cells that lie behind the seal receive little or no oxygen and nutrients, which ultimately causes them to die.

Since the brain is only able to regenerate nerve cells to a very limited extent, it cannot replace the dead cells: the brain is irreversibly damaged. The occlusion of various blood vessels can contribute to the development of Weber syndrome. In the vertebral artery, blood flows not only to the brain, but also to the dura mater and the spinal cord.

The basilar artery is one of its branches, which also divides into different branches. One of these, the posterior cerebral artery, can also cause Weber syndrome if it is blocked. Regardless of the blood vessel affected, the blockage is often caused by blood clots.

Deposits in the blood vessels can form thrombi, which, moreover, often tear themselves away and get stuck in constrictions or in thinning blood vessels. Such embolisms can also be based on droplets of fat blocking the blood vessel.

Symptoms, ailments & signs

On the side of the brain damage (ipsilateral), Weber syndrome typically results in oculomotor paresis: the oculomotor nerve is interrupted and can therefore no longer transmit nerve signals to the innervated eye muscles. In the case of complete oculomotor paralysis, the ipsilateral eye hangs down and the gaze is directed outwards.

Visual disturbances include forced pupil dilation (mydriasis), drooping of the eyelid (ptosis), impaired pupillary response, and accommodation disorders. There are two possible sub-forms of partial oculomotor paralysis: internal and external paralysis.

The former manifests itself in mydriasis and accommodation disorders, while in external paralysis the affected eye is turned downwards and outwards. One-sided paralysis (hemiparesis) on the side opposite the brain damage (contralateral) is one of the characteristic symptoms of Weber syndrome.

Depending on how severely the brain is damaged, the paralysis can take different forms. It often appears as spastic paralysis and is accompanied by dystaxias. Other symptoms depend on the affected nerves in the midbrain.

Diagnosis & course of disease

As part of the diagnosis, doctors use imaging methods, for example computed tomography (CT) or magnetic resonance tomography (MRT). Both allow the affected tissue to be precisely located and the extent of the damage to be assessed. In many cases, neurological tests carried out beforehand can already provide initial information about the nature of the disease. In addition, the attending physician clarifies, depending on the cause, whether other organs are affected or endangered.

Complications

The Sturge Weber Syndrome promotes malformations in the facial area. Those affected suffer from severe aesthetic impairments, which leads to psychological complaints. Inferiority complexes are common in patients and self-esteem decreases rapidly. The level of suffering is particularly high at a young age, as many patients are victims of bullying.

Furthermore, the syndrome entails limited sensitivity: numbness, indefinable pain, and tingling and burning sensations that cannot be localized, worsen the suffering. This is made even more difficult by paralysis in various parts of the body. Cataracts often form in connection with Sturge-Weber syndrome and the lens of the eye is significantly clouded.

A high sensitivity to light is also a sign that the person has a cataract. Often, glaucoma occurs, which increases the pressure in the eye to the point that the patient can go blind. Intellectual disabilities and developmental delays are also not uncommon, although it makes little difference whether or not Sturge-Weber syndrome is treated. There are also emotional behavior disorders and learning disorders.

It is seldom possible to carry out physical activities without outside help, which limits the patient's independence and mobility. Severe headaches are common in Sturge-Weber syndrome and further reduce the quality of life of those affected. The blood vessels in the brain develop abnormally, and severe seizures and epilepsy make the state of health worse.

When should you go to the doctor?

The person affected should definitely consult a doctor with Weber syndrome. It is a serious condition that definitely needs to be examined and treated by a doctor. The earlier the disease is recognized and treated, the better the further course of the disease will usually be. In most cases, aesthetic impairments or malformations in the face indicate Weber syndrome. The disease can also significantly increase the risk of a stroke. If a stroke occurs, an emergency doctor should be called immediately or a hospital should be visited.

Likewise, intellectual impairments in the person concerned can indicate this syndrome and must be examined by a medical professional. The tumors on the face often lead to failures in the visual field or very severe headaches. First and foremost, a general practitioner can be seen in Weber syndrome. The further examination is usually carried out in a hospital. Since the syndrome can lead to severe psychological upset or depression, psychological treatment should also be carried out in any case. This disease may also reduce the life expectancy of the person affected.

Treatment & Therapy

As a first therapeutic measure, doctors ensure immediate care for the patient. An imaging test also shows whether intravenous thrombolysis or some other immediate measure is possible. In some cases, this procedure allows the occlusion of the blood vessel to be removed shortly after an ischemic stroke, thereby preventing further tissue death.

However, numerous factors influence its success and there is a risk of bleeding from intravenous thrombolysis. Intraarterial lysis with a catheter (catheter intervention) may also be considered. If the cause of Weber syndrome is not a clogged blood vessel but a bleeding, surgical interventions are an option that help to relieve the tissue.

After an ischemic stroke, around 40 percent of those affected die in the first year. In the long term, patients undergo extensive rehabilitation, which includes occupational therapy and physiotherapy, speech therapy, (neuro-) psychological and psychiatric treatments. The aim of these measures is to maintain the patient's independence and remaining abilities as much as possible.

In some cases, brain cells from other areas that are still intact can take over the tasks of the dead cells if the therapy actively stimulates and encourages them. Long-term treatment also includes the prevention of further strokes.

prevention

Prevention of Weber syndrome is essentially the same as general stroke prevention, as this is the cause of the brain damage in most cases. In this context, the treatment of other diseases such as diabetes mellitus, lipid metabolism disorders, high blood pressure and others is crucial.

Lifestyle factors that each person can influence themselves include alcohol consumption, smoking habits, diet, and exercise. Avoiding stress and being overweight can also help prevent strokes.

Aftercare

With Weber syndrome, those affected usually have only limited and only a few direct follow-up measures available, so that those affected should consult a doctor very early on in this disease. The earlier a doctor is contacted, the better the further course of this disease will usually be.

It is also a congenital disease, so that it cannot be completely cured. For this reason, the person affected should have a genetic examination and counseling carried out, even if they want to have children, in order to prevent the recurrence of Weber syndrome in their descendants. As a rule, various surgical interventions are necessary in order to limit the symptoms correctly and permanently.

In any case, the person affected should rest and take care of himself after such an operation, whereby no strenuous or stressful activities should be carried out. The support and care of one's own family is also very important with this disease. This can also prevent psychological upsets and depression. The further course of the syndrome depends very much on the exact form of the disease, although in some cases the life expectancy of the person affected is reduced.

You can do that yourself

If Weber syndrome is suspected, the doctor must first be consulted. The rare condition occurs as a result of severe brain disorders, which is why a quick diagnosis is necessary. It is best to see a doctor as soon as the first typical symptoms appear.

After the diagnosis, depending on the symptoms, an individual therapy can be worked out that can be supported by those affected and their relatives. Physiotherapy can be supported at home through independent training. Endurance sports and stretching are important, the exact measures depending on the symptoms. The training plan should be drawn up together with the responsible physiotherapist.

Weber syndrome can lead to a wide variety of secondary diseases. For example, epileptic seizures can occur. The relatives and the person affected must take the necessary measures to reduce the risk of falling or injury to a minimum. The tissue damage can also lead to motor disorders, which in turn are treated by extensive physiotherapy. The self-help measures must be regularly adapted to the varying course of the disease in order to continue to improve the state of health.